Neurology 2002
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NEUROLOGY Dr. J. Miyasaki and Dr. C. Jaigobin Daniel Lebovic and Dorothy Lo, chapter editors Geena Joseph, associate editor INTRODUCTION . 3 CRANIAL NERVES . .25 Cranial Nerve I (Olfactory) BASIC NEUROANATOMY . 4 Cranial Nerve II (Optic) Cranial Nerve III (Oculomotor) DIAGNOSTIC INVESTIGATIONS . 7 Cranial Nerve IV (Trochlear) CT Cranial Nerve V (Trigeminal) MRI Cranial Nerve VI (Abducens) Other Investigations Cranial Nerve VII (Facial) Lumbar Puncture Cranial Nerve VIII (Vestibulocochlear) Cranial Nerve IX (Glossopharyngeal) SEIZURE DISORDERS AND EPILEPSY . 8 Cranial Nerve X (Vagus) Classification of Seizures Cranial Nerve XI (Accessory) Clinical Approach to Seizures Cranial Nerve XII (Hypoglossal) Types of Seizures Status Epilepticus NEURO-OPHTHALMOLOGY . .31 Visual Field Defects ALTERED LEVEL OF CONSCIOUSNESS . .13 Disorders of Lateral Gaze Approach to Altered Level of Consciousness Optic Disc Edema COMA Transient Monocular Blindness Brain Death Pupillary Signs Persistent Vegetative State Nystagmus BEHAVIOURAL NEUROLOGY . .16 VERTIGO . .35 Acute Confusional States Dementia GAIT DISTURBANCES . .36 Causes of Dementia Aphasia (Dysphasia) CEREBELLAR DISORDERS . .36 Apraxia Functional Anatomy of the Cerebellum Agnosia Symptoms and Signs of Cerebellar Disease Acquired Cerebellar Diseases MOVEMENT DISORDERS . .21 Hereditary Ataxias Clinical Features Differential Diagnosis of Ataxia Neuronal Connections of the Basal Ganglia Parkinson’s Disease DISEASES OF THE SPINAL CORD . .38 “Parkinson Plus” Disorders Clinical Features Tremor Spinal Cord Syndromes Chorea Motor Neuron Diseases Dystonia Spinal Root Myoclonus Tics PERIPHERAL NEUROPATHIES . .40 Approach to Peripheral Neuropathies MCCQE 2002 Review Notes Neurology – N1 NEUROLOGY . CONT. FOCAL AND MULTIFOCAL NEUROPATHY . 41 STROKE . .. .49 Myelinopathies Classification Axonopathies Stroke Terminology Neuronopathies Making the Complete Diagnosis: “Four Questions” Diabetic Polyneuropathies MULTIPLE SCLEROSIS . .. .53 NEUROMUSCULAR JUNCTION . 43 DISORDERS CNS INFECTIONS . .. .54 Myasthenia Gravis Meningitis Lambert-Eaton Syndrome Encephalitis Intracranial Abscess MUSCLE DISEASES. 44 Polymyositis/Dermatomyositis NEUROLOGIC COMPLICATIONS OF . 57 Metabolic Myopathies SYSTEMIC DISEASES Inherited Muscle Diseases Metabolic Diseases Endocrine Diseases HEADACHE . 46 Collagen Vascular Diseases Migraine Tension-Type Headache REFERENCES . .. .60 Cluster Headache Medication-Induced Headache Traction Headache Meningeal Irritation Giant Cell Arteritis N2 – Neurology MCCQE 2002 Review Notes INTRODUCTION When approaching a patient with a neurologic disorder always ask yourself: ❏ where is the lesion? • cerebrum • cerebellum • brainstem • spinal cord • nerve root • peripheral nerve • neuromuscular junction • muscle • not confined to one level ❏ what is the cause of the lesion? • Vascular • Infectious • Neoplastic • Degenerative • Inflammatory-immunologic • Congenital-developmental • Autoimmune • Toxic/ traumatic • Endocrine/ metabolic ❏ is the lesion focal, multifocal or diffuse? Table 1. Temporal and Spatial Features of the Major Disease Categories Acute Subacute Chronic Focal Vascular (e.g. infarct, Inflammatory Neoplasm intraparenchymal hemorrhage) (e.g. abscess, myelitis) Diffuse Toxic Inflammatory Degenerative Metabolic (e.g. anoxia) (e.g. meningitis, encephalitis) Table 2. An Anatomic Approach to Neurologic Disorders, Symptoms and Signs Location Disorders Symptoms Signs Cerebrum Seizure disorders Aphasia, seizures Gaze preference Coma Involuntary movements Cortical blindness and Confusion Visual field defects sensory loss Dementia Cognitive/personality changes Homonymous field defects Aphasia Neglect, apraxia, Movement disorders Anosognosia Cerebellum Cerebellar degeneration Clumsiness Tandem gait impairment Lack of coordination Dysdiadochokinesis Unsteadiness Abnormal heel-shin, Vertigo finger-nose, nystagmus Brainstem Cranial nerve palsies Diplopia, dizziness, deafness Cranial nerve abnormalities Dysarthria, dysphagia UMN lesions (bilateral) Decreased strength/sensation Sensory loss (crossed) in face and body Nystagmus Vertigo Spinal Cord Spinal cord syndromes Sensory level Upper motor neuron (UMN) signs Amyotrophic lateral Distal weakness Loss of superficial reflexes sclerosis (ALM) Bowel and bladder changes Nerve Root Nerve root compression Same as peripheral nerve + Weakness in myotomal group pain (sharp, electric, radiating) Sensory loss in dermatome Peripheral Neuropathies Distal weakness Normal or decreased tone Nerve with sensory change, atrophy Decreased reflexes Neuromuscular Myasthenia gravis Proximal symmetric weakness Repeated strength testing Junction Lambert-Eaton syndrome No sensory loss to elicit fatigability Fatigable weakness Muscle Polymyositis (PMY) Proximal symmetric weakness Normal/ decreased tone Muscular dystrophies No sensory loss Normal/ decreased reflexes Metabolic Minimal atrophy Structural myopathies Disorders not Headache confined to Stroke one level Multiple sclerosis (MS) CNS infections HIV/AIDS Alcohol MCCQE 2002 Review Notes Neurology – N3 BASIC NEUROANATOMY oculomotor nerves corticospinal and corticobulbar tracts substantia nigra red nucleus oculomotor medial and spinal lemnisci nucleus cerebral aqueduct (of Sylvius) superior colliculus Figure 1. Section through the Midbrain at the Level of the Superior Colliculus decussation of the superior cerebellar peduncle (branchium conjunctivum) substantia nigra corticospinal, corticobulbar tracts medial and trochlear nucleus (IV) spinal lemnisci cerebral aqueduct (of Sylvius) inferior colliculus Figure 2. Section through the Midbrain at the Level of the Inferior Colliculus groove for basilar artery abducens nerve (VI) corticospinal, corticobulbar tracts medial lemniscus spinal lemniscus facial nucleus (VII) trigeminal nucleus (V) abducens nucleus (VI) middle cerebellar peduncle vestibular nucleus (VIII) Figure 3. Section through the Pons corticospinal tracts (pyramids) hypoglossal nerve (XII) olive spinal lemniscus medial lemniscus nucleus ambiguus vagus nerve (X) spinal trigeminal middle nucleus cerebellar peduncle tract of the spinal trigeminal nucleus hypoglossalg nucleusnucleus nucleus solitarius vestibular nucleus dorsal vagal nucleus fourth ventricle Figure 4. Section through the Open Medulla Illustrations by Dr. P. Stewart N4 – Neurology MCCQE 2002 Review Notes BASIC NEUROANATOMY . CONT. corticospinal tracts (pyramids) spinal lemniscus central canal origin of medial lemniscus spinal trigeminal nucleus tract of spinal nucleus cuneatus trigeminal nucleus fasciculus cuneatus nucleus gracilis fasciculus gracilis Figure 5. Section through the Closed Medulla upper motor neurons in motor cortex internal decussation of the capsule pyramids pyramids lateral corticospinal tract medial corticospinal tract limb muscles lower motor neuron axial muscles axial muscles Figure 6. Corticospinal Motor Pathway sensory cortex (lower limb & trunk) sensory cortex (upper limb) thalamus internal capsule medial lemniscus nucleus cuneatus internal arcuate fibers fasciculus nucleus gracilis input from upper limb dorsal root fasciculus gracilis ganglion input from lower limb & trunk Figure 7. Discriminative Touch Pathway from Body Illustrations by Dr. P. Stewart MCCQE 2002 Review Notes Neurology – N5 BASIC NEUROANATOMY . CONT. sensory cortex third-order sensory neuron spinal lemniscus spinothalamic tract dorsal root ganglion first-order sensory neuron second-order sensory neuron within 1-2 spinal levels of their entry, axons of first order neurons synapse onto second order neurons, whose axons then decussate before ascending as the spinothalamic tract Figure 8. Spinothalamic Pain Pathway from Body thalamus internal capsule internal thalamus capsule sensory cortex face region sensory cortex face region trigeminal ganglion trigeminal ganglion spinal lemniscus medial lemniscus input from face (trigeminal lemniscus) input from face (trigeminal lemniscus) tract of the spinal trigeminal nucleus chief sensory trigeminal nucleus spinal trigeminal nucleus Figure 9. Discriminative Touch Pathway Figure 10. Spinothalamic Pain Pathway from Face from Face Illustrations by Dr. P. Stewart N6 – Neurology MCCQE 2002 Review Notes DIAGNOSTIC INVESTIGATIONS CT X-Rays Attenuated in Proportion to the Density of Tissue ❏ black: air, fat, CSF, water ❏ gray: edematous or infarcted brain, normal brain, subacute hemorrhage (5-14 days) ❏ white: acute hemorrhage (hemoglobin), IV contrast, bone, metal ❏ CT with contrast is useful in detecting breakdown of Blood-Brain-Barrier, and in conditions such as neoplasm, abscess, vascular malformation Clinical Pearl ❏ CT with no contrast – bleeds, infarctions. ❏ CT with contrast – tumours, abscesses, vascular malformations. MRI ❏ better than CT in the evaluation of brainstem (posterior fossa), spinal cord lesions ❏ more sensitive for pathology Advantage Black Gray White T1-weighted Anatomy CSF, bone, often Normal brain Fat, subacute hemorrhage tumour/infarction T2-weighted Pathology Bone Normal brain CSF, brain edema, infarction, tumour ❏ other MR images - proton density, diffusion, flair ❏ high velocity blood flow appears black on both T1 and T2, so intracranial blood vessels can be imaged ❏ good at differentiating periventricular pathology (e.g. white matter demyelination) from CSF ❏ MR angiography adequate for large-scale vascular lesions OTHER INVESTIGATIONS Table 3. Other Techniques of Neuroimaging Imaging Technique Basic Principle Clinical Application MRA (Magnetic resonance Special pulse sequences for blood Visualization